Abstract
The authors described a patient with primary non-familial amyloidosis who showed remarkable peripheral and autonomic neuropathy. Sixty five years old man developed orthostatic hypotension, severe polyneuropathy, nephrotic syndrome and cardiomegary 20 years after hypotension was noted. He died of sinus arrest despite of the treatment with dimethylsulfoxide (DMSO).
1. Peripheral nerve biopsy and autopsy revealed that Congo red positive and potassium permanganate resistant fibril was abundant deposited in interstitial space in multiple organs.
2. Amyloid protein isolated from the kidney was chiefly composed of the molecular weight of 10 kilodalton, and was identified as AL (λ) type.
3. The immunological tests showed decreased amount of IgG, IgA and IgM, poor response of lymphocytes to PHA, Con A mitogens, and increased ratio of OKT 4+/OKT 8+T cells.
While polyneunopathy is one of the common clinical features in familial amyloidosis, only 14% of the patients with primary non-familial amyloidosis have this manifestation. Properties of the amyloid protein was probably related to immunological and neurological abnormalities in this patient.
